Müllerian Anomalies

Congenital Abnormalities that Affect the Female Reproductive System

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During pregnancy, Müllerian ducts in a developing fetus undergo changes to become the mature female reproductive tract. When they do not properly develop, the following conditions, known as Müllerian anomalies, may be present at a child’s birth:

  • Vaginal abnormalities -- the baby is born without a vagina (vagina agenesis) or with a blockage or obstruction in the vagina (vaginal septum).
  • Uterine abnormalities -- the uterus is separated into two parts by muscle or tissue, the uterus is heart-shaped (one uterus with two cavities), or a double uterus is formed (two uteri and two cervixes).
  • Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome -- the baby has an undeveloped uterus and upper vagina with external genitalia that appear normal.

These congenital abnormalities may be identified early in a child’s life. However, it is common to delay treatment until they are psychologically ready and able to perform self-care. Identifying and correcting these conditions is our goal. Our team of specialists helps you understand your condition and your options to improve your quality of life.

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Diagnosing Müllerian Anomalies

Vaginal agenesis, vaginal septum, and congenital uterine abnormalities often go undetected and may cause no symptoms. However, that may change at the start of menses or sexual activity, when trying to become pregnant, or during pregnancy. It’s at that point that either menstruation doesn’t start or a woman experiences pain and difficulty having sex, getting pregnant, or maintaining a pregnancy.

Working with a Specialist
Some pediatricians and gynecologists may not consider a congenital difference in anatomy when a teen complains of abdominal pain that could be related to blocked menstrual flow, or a woman has difficulty having intercourse. Getting your care from a specialist who works with young women with these concerns will ensure you get the right treatment at the right time. Coming to terms with these anatomical differences and preparing for the self-care needed are all important steps in the treatment process.

Our Team Approach
Our team includes experts in pediatric and adolescent gynecology, urogynecology, and other specialties, including urology, physical therapy, reproductive endocrinology, and behavioral health. That’s important when complicated anatomical differences require treatment. These specialists may be needed when conditions that affect the kidneys, ureters, and bowels are also present. Our patient navigator will help you coordinate multiple appointments with specialists as needed and will help you manage the process from your initial consultation through your diagnosis and treatment.

Our Locations

Duke Health offers locations throughout the Triangle. Find one near you.

Imaging and Other Tests

You may be sent for imaging tests before your first appointment so you can discuss the results with your doctor during your initial consultation. Your doctor may also perform an internal and external pelvic exam during your first visit. Additional imaging may be requested after the physical exam. Some of these imaging tests can take place in your doctor’s office. Depending on your condition, you may undergo one or more of the following imaging tests.

Pelvic Ultrasound

A wand-like device uses high-frequency sound waves to produce images. It may be moved over the abdominal area or inserted into the vagina or rectum to examine the uterus and ovaries.

Hysterosalpingogram (HSG)

X-ray images are used to evaluate your fallopian tubes and the inside of your uterus. A thin tube that is threaded through the vagina and cervix, and a contrast dye is injected into the uterus through the tube. The dye helps show the outline of the fallopian tubes and can help your doctor identify a blockage or abnormality.

Sonohysterogram (SHG)

Ultrasound is performed to evaluate your uterus and the shape of your uterine cavity. A thin tube is inserted through the vagina and cervix, and s saline solution is put in the uterus through the tube. The solution fills the uterus and allows your doctor to see any abnormalities in the uterus.

Magnetic Resonance Imaging (MRI)

MRIs use a powerful magnet, radio waves, and an advanced computer system to produce detailed pictures of organs, bones, and tissues. It is used to confirm a diagnosis and identify other conditions that may be present.

Blood Tests

These may be performed to check for differences in sex chromosomes.

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Psychological Readiness

It is important that you are psychologically ready before pursuing treatment. Often, young girls experience different emotions when they are anatomically different from their peers. Part of the evaluation includes a discussion about psychological readiness. Your doctor may recommend you speak to a social worker or receive therapy from a behavioral health specialist. They can help you come to terms with your condition, have candid discussions with your partner, and get ready for treatment, including surgery that requires extensive self-care.

Best Hospital for Obstetrics and Gynecology in North Carolina

Where you receive your care matters. Duke University Hospital is proud of our team and the exceptional care they provide. That is why our obstetrics and gynecology program is nationally ranked, and is the highest-ranked program in North Carolina, according to U.S. News & World Report for 2023-2024.

Treatment Options

Non-surgical and surgical treatment options are available and may be recommended as part of your personalized treatment plan. If surgery is recommended, it may be performed vaginally or through small incisions in your abdomen.

Vaginal Dilatation

Dilator treatment is often the first treatment recommended. It does not require surgery and may be prescribed to create an indent in the vagina when MRKH or vaginal agenesis is present. A hard, smooth, plastic, tampon-shaped device is applied daily for 20 to 30 minutes with hand pressure to stretch the vaginal canal. This process must be done regularly or the indent will disappear.


Surgery to create a functional vagina may be performed when a young woman wants a longer, more functional vagina. An opening is made in the vaginal area. A graft of skin is taken from the inside of the cheek, buttocks, or leg and placed inside the opening. A mold is also placed inside to maintain the vaginal canal during healing. The mold is later removed. Regular use of vaginal dilation and sexual intercourse helps to maintain the shape.

Vaginal Septum Surgery

When a wall of tissue, or a septum, forms an obstruction in the vagina, surgery may be performed to remove the tissue to form a single vaginal canal. The septum can form horizontally or vertically. It can block part or all of the vaginal canal. Recovery and rehabilitation following the surgery may include vaginal dilation.


In most cases, a double or heart-shaped uterus does not require surgery. However, if it impacts your ability to get pregnant or maintain a pregnancy, a partial hysterectomy may be performed to remove the extra uterus and leave one functioning uterus.

Physical Therapy

A physical therapist who specializes in the pelvic floor may work with you to help manually stretch vaginal tissues or relax painful muscles. This therapy may be recommended if you are having trouble participating in sexual intercourse.

This page was medically reviewed on 09/09/2021 by